Pulmonary hypertension in connective tissue diseases, new evidence and challenges
SourceEuropean Journal of Clinical Investigation, 51, 4, (2021), article e13453
Article / Letter to editor
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European Journal of Clinical Investigation
SubjectRadboudumc 16: Vascular damage RIHS: Radboud Institute for Health Sciences; Radboudumc 5: Inflammatory diseases RIHS: Radboud Institute for Health Sciences
Pulmonary arterial hypertension is a lethal complication of different connective tissue diseases such as systemic sclerosis, mixed connective tissue disease and systemic lupus erythematosus. Although the treatment possibilities for patients with pulmonary arterial hypertension have increased in the last two decades and survival of patients with idiopathic pulmonary arterial hypertension has improved, the latter is not the case for patients with pulmonary arterial hypertension associated with connective tissue disease. In this narrative review, we review recent literature and describe the improvement of early diagnostic possibilities, screening modalities and treatment options. We also point out the pitfalls in diagnosis in this patient category and describe the unmet needs and what the focus of future research should be.
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